Increased incidence of autoimmune markers in patients with combined pulmonary fibrosis and emphysema

被引：50

作者：

Tzouvelekis, Argyris ^{[1
]}

Zacharis, George ^{[1
]}

Oikonomou, Anastasia ^{[2
]}

Mikroulis, Dimitrios ^{[3
]}

Margaritopoulos, George ^{[4
]}

Koutsopoulos, Anastasios ^{[5
]}

Antoniadis, Antonis ^{[6
]}

Koulelidis, Andreas ^{[1
]}

Steiropoulos, Paschalis ^{[1
]}

Boglou, Panagiotis ^{[1
]}

Bakali, Matina ^{[7
]}

Froudarakis, Marios ^{[1
]}

Bouros, Demosthenes ^{[1
]}

机构：

[1] Democritus Univ Thrace, Univ Hosp Alexandroupolis, Dept Pneumonol, Alexandroupolis 68100, Greece

[2] Democritus Univ Thrace, Univ Hosp Alexandroupolis, Dept Radiol, Alexandroupolis 68100, Greece

[3] Democritus Univ Thrace, Univ Hosp Alexandroupolis, Dept Cardiothorac Surg, Alexandroupolis 68100, Greece

[4] Gen Hosp Kavala, Dept Pneumonol, Kavala, Greece

[5] Democritus Univ Thrace, Univ Hosp Alexandroupolis, Dept Pathol, Alexandroupolis 68100, Greece

[6] Gen Hosp Serres, Dept Pneumonol, Serres, Greece

[7] Democritus Univ Thrace, Univ Hosp Alexandroupolis, Dept Microbiol, Alexandroupolis 68100, Greece

来源：

BMC PULMONARY MEDICINE | 2013年 / 13卷

关键词：

CONNECTIVE-TISSUE DISEASE; INTERSTITIAL LUNG-DISEASE; MICROSCOPIC POLYANGIITIS; COPD; CLASSIFICATION; ASSOCIATION; CRITERIA; AUTOANTIBODIES; PATHOGENESIS; EXPRESSION;

D O I：

10.1186/1471-2466-13-31

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

Background: Combined pulmonary fibrosis and emphysema (CPFE) is an umbrella term encompassing upper lobe emphysema and lower lobe pulmonary fibrosis with pathogenesis elusive. The aim of our study was to investigate the incidence of autoimmune markers in patients with CPFE. Methods: In this multicenter study we retrospectively evaluated records from patients with CPFE (n=40) and IPF (n=60) without emphysema. Baseline demographic characteristics, high-resolution computed tomography (HRCT), spirometry, histopathological, treatment, serum immunologic and survival data were investigated. B cell presence was estimated with CD20 immunostaining in representative lung biopsy samples from CPFE patients and control subjects. Results: A statistically significant increased number of CPFE patients with elevated serum ANA with or without positive p-ANCA titers compared to patients with IPF without emphysema was observed. Patients with CPFE and positive autoimmune markers exhibited improved survival compared to patients with a negative autoimmune profile. A massive infiltration of clusters of CD20+ B cells forming lymphoid follicles within the fibrotic lung in CPFE patients with positive serum immunologic profile compared to patients with negative profile, was noted and positively correlated with improved survival. Conclusions: A significant proportion of patients with CPFE may present with underlying auto-immune disorders that may reside insidiously and be associated with favorable prognosis. Early identification of these patients using a panel of auto-antibodies may lead to more targeted and effective therapeutic applications.

引用

页数：11

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